Surgical management of pediatric Cushing's disease

Abstract

Background: Cushing's disease may have a direct effect on growth pattern, pubertal maturation, and long-term survival in pediatric patients.

Methods: Dexamethasone suppression test was done in 10 children (median age, 15 years) and showed variable suppressibility, with microadenoma seen in 5, macroadenoma in 3, and normal gland in 2 patients. Inferior petrosal sinus sampling (IPPS) was also carried out and confirmed pituitary adenoma as source of ACTH in 2 patients. We adopted sublabial transsphenoidal (n = 9) or pterional transsylvian route (n = 1) in macroadenoma with conchal sphenoid sinus. Serum cortisol level <50 nmol/L was taken as the criteria for biochemical remission.

Results: Clinical remission was achieved in 7 of 10 operated patients. In 2 patients where clinical remission was achieved, postoperative BSC could not be done. Postoperative BSC was less than 50 nmol/L in 2 (25%) of 8 patients and remained elevated in 6. Remission was achieved in both patients with postoperative BSC less than 50 nmol/L and in 3 of 6 with elevated levels. Three patients had neither clinical nor biochemical remission: 2 underwent bilateral adrenalectomy and 1 received radiotherapy. Postoperative CSF leak seen even in microadenomas associated with arachnoidal prolapse. Among 7 patients who initially remitted (median follow-up of 82 months; range, 24-120 months), recurrence of disease occurred in 3 (42.8%) patients after a median interval of 5 years.

Conclusions: In children with CD, endocrinal manifestations are more frequent than visual symptoms. Transsphenoidal route is the preferred approach, but a nonpneumatised sphenoid sinus may be present. Sellar arachnoidal prolapse may cause postoperative CSF leak even in microadenomas. Surgery is the first line of treatment, but constant monitoring is mandatory to pick up the relapsed cases.